Stroke and skin rash: A rare case of Henoch-Schonlein purpura.

Henoch-Schonlein purpura (HSP) is a small vessel vasculitis that is rare in adults. Here, we present a case of a woman who presented with palpable purpura, abdominal pain, arthritis and ischemic stroke. The patient met the diagnostic criteria of HSP. However, cerebrovascular disease is reported as an uncommon, yet fatal, complication of HSP. The patient responded to aggressive immunosuppression with pulses of corticosteroids and cyclophosphamide. In the absence of an established protocol of treatment of such neurologic emergency in HSP patients, this report demonstrates a successful outcome.

Introduction

Henoch-Schonlein (HSP) is a small vessel vasculitis that involves joints, skin, kidneys and gastrointestinal tract.[1] It commonly affects children and young adults and presents classically with palpable purpura, abdominal pain and joint symptoms. Adults are less affected by HSP, although complications are more common in this age group.[2] The central nervous system (CNS) is rarely involved in HSP.[3] As CNS affection is more likely in other vasculitis syndromes, it is necessary to exclude these causes. CNS manifestations include seizures,[4] stroke, visual abnormalities as well as speech disturbances.[3] Patients who develop stroke can have either multiple infarcts or hemorrhage, and may have raised anti-phosphatidylethanolamine antibody in serum.[5] Usually, these patients do not have hypertension. On the other hand, hypertensive patients are reported to have a reversible CNS dysfunction.[6] Neuroimaging studies in patients of HSP with CNS features show a predominant pattern of infarcts affecting two vessel territories, and these patients require aggressive immunosuppression.[3]

Case Report

A 46-year-old lady was admitted for sudden onset of weakness of the right side of the body and facial deviation without any history of unconsciousness, headache, vomiting or seizure. She was diagnosed as a case of hypertension 15 days back, for which she was given metoprolol 25 mg/day. In the emergency room, examination revealed stable vitals, blood pressure 170/110 mmHg, Glasgow coma score 12, right-sided hemiparesis and left-sided upper motor neuron facial nerve palsy. She also had palpable purpura over both the shins and buttocks and a digital infarct of the left thumb. Systemic examination was otherwise normal.

The patient had developed swelling and redness of both the knees and ankles 3 weeks prior to admission, which was associated with severe abdominal pain. She was prescribed corticosteroids for 1 week, which she stopped after relief of symptoms. Soon after withdrawal of the drug, she developed gradual darkening of the tip of the left thumb and painful crops of reddish lesions appeared over her legs and buttocks [Figure 1].

Figure 1

Dry gangrene of the left thumb due to vasculitis

On admission, she underwent routine hematologic and biochemical testing and a noncontrast computed tomography (CT) scan of the brain. There was normochromic normocytic anemia, leukocytosis, raised erythrocyte sedimentation rate (ESR) and normal platelet count [Table 1]. Her blood urea nitrogen and creatinine was mildly elevated, while other biochemical parameters remained within normal limits. CT brain showed multiple infarcts in the left internal capsule, basal ganglia and right thalamus [Figure 2]. A routine urinalysis revealed presence of albumin and active urine sediment of red blood cells (RBC) and RBC casts. Estimation of 24-h urinary protein showed nonnephrotic range proteinuria [Table 1]. Ultrasonography of the kidneys showed normal kidney size with increased cortico-medullary echogenecity. Tests for antinuclear factor, anti-neutrophil cytoplasmic antibody (p- and c-ANCA ) and anti-phospholipid antibody were negative. A punch biopsy was taken from the skin lesions over the legs, which revealed leucocytoclastic vasculitis [Figure 3].

Table 1

Summary of laboratory tests

Figure 2

Noncontrast computed tomography scan of the brain. Arrowheads show bilateral basal ganglia infarcts

Figure 3

Skin biopsy from the palpable purpura site, stained with hematoxylin and eosin, ×10 magnification, and showing leucocytoclastic vasculitis. Arrowheads indicate neutrophils infiltrating the vessel wall

The patient was started on low-dose aspirin, metoprolol and ramipril. She remained conscious, oriented and afebrile, without new neurological deficits, and maintained normal urine output. However, on the fourth day of hospital stay, there was onset of abdominal pain and inflammation of both knee joints.

The clinical features and biopsy evidence supported the diagnosis of HSP, although age and presentation of the patient were not typical of the disease. Considering the presence of both renal and cerebrovascular involvement, the patient was administered intravenous cyclophosphamide 500 mg/sq meter on Day 1 combined with methylprednisolone 1 gm/day for Days 1–3. This was followed by a maintenance dose of prednisolone 0.5 mg/kg/day.

The patient was readmitted after 1 month for follow-up and a repeat pulse of cyclophosphamide and methylprednisolone. On clinical examination, her paresis had improved. Routine investigations of blood and urine showed an improvement of anemia and absence of active urinary sediment. She is scheduled to receive four more monthly pulses of cyclophosphamide and methylprednisolone along with maintenance dose of prednisolone.

Discussion

According to the American College of Rheumatology criteria (1990), diagnosis of HSP requires presence of two or more of the following criteria: age less than 20 years, palpable purpura, acute abdominal pain and presence of granulocytes in the walls of small vessels. Although our patient is 46 years old, she meets two of these criteria. She was therefore diagnosed as a case of HSP.

Apart from classical involvement of skin, kidney, gut and joints, complications affecting the CNS have rarely been reported. Cerebrovascular accident is a rare complication HSP, which is mainly described in children and young adults.[78] But, adult patients with HSP suffering from cerebrovascular accident is extremely rare. Karamadoukis et al.[9] and noneLévaif F et al.[10] described adult-onset HSP with intracerebral hemorrhage as a rare complication. To the best of our knowledge, there is only one other published report of cerebral infarction in a patient of HSP.[11] Acute rise of blood pressure has been considered to be the primary mechanism of intracerebral hemorrhage.[12] Other possible causes include the presence of cerebral vasculitis and reduced levels of factor XIII[13] and prothrombin.[14] However, the etiology behind cerebral infarction remains obscured till date. The management of cerebral infarction in older HSP patient lacks proper guideline as the reported incidence is low. Intravenous pulse methylprednisolone followed by oral steroids has been shown to be effective in the management of severe HSP nephritis.[15] In our patient, both HP and its neurological symptoms were successfully treated with intravenous cyclophosphamide and methylprednisolone, followed by oral prednisolone. In conclusion, we present a case of HSP with an atypical presentation of stroke in an adult female that responded to immunosuppression with cyclophosphamide in addition to steroid therapy.